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Plasma zinc status, growth, and maturation in children with sickle cell disease
M B Leonard1, B S Zemel, D A Kawchak
1Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, 19104, USA.
Insights
Low plasma zinc (Zn) is common in children with sickle cell disease (SS genotype). This deficiency is linked to impaired growth, muscle mass, and delayed skeletal and sexual maturation.
Area of Science:
- Pediatric Hematology
- Nutritional Biochemistry
- Growth and Development
Background:
- Sickle cell disease (SS genotype) is a genetic blood disorder affecting red blood cells.
- Nutritional status, particularly micronutrient levels like zinc, can impact growth and development in chronic diseases.
- Understanding zinc's role is crucial for managing growth complications in pediatric sickle cell disease patients.
Purpose of the Study:
- To investigate the relationship between plasma zinc status and physical growth in children with SS genotype sickle cell disease.
- To assess the association of plasma zinc levels with skeletal and sexual maturation in this pediatric population.
Main Methods:
- A cross-sectional study involving 104 children (0.4–18 years) with SS genotype sickle cell disease.
- Measurements included plasma zinc concentration (Znp), anthropometrics (height, weight, etc.), and body composition.
- Skeletal maturation was assessed via hand-wrist X-rays, and sexual maturation using Tanner stages.
Main Results:
- 44% of patients exhibited low plasma zinc levels (<10.7 micromol/L).
- Low Znp was significantly associated with reduced height, weight, upper arm muscle area, fat-free mass, and elbow breadth.
- Children with low Znp showed delayed skeletal maturation and, in older children, decreased pubic hair and genital development (Tanner stages).
Conclusions:
- Decreased plasma zinc is prevalent in children with SS genotype sickle cell disease.
- Low zinc status is correlated with impaired linear growth, reduced muscle mass, and delayed skeletal and sexual maturation.
- Zinc status is a significant factor influencing growth and development in children with sickle cell disease.
Objective:
The objective of this study was to determine the relation of plasma zinc (Zn) status to growth and maturation in children with SS genotype sickle cell disease.
Study Design:
A cross-sectional study of 104 subjects who were 50% female and ranged in age from 0.4 to 18 years was performed. Measures included plasma Zn concentration (Znp), height, weight, skinfold thicknesses, elbow breadth, upper arm muscle area, and fat-free mass and fat mass by total body electrical conductivity. Skeletal maturation was assessed by hand-wrist x-ray evaluation and sexual maturation by Tanner stage.
Results:
A total of 44% of the patients had low Znp (<10.7 micromol/L [70 microg/dl]); those with low Znp had significantly lower SD scores for height (p = 0.003), weight (p = 0.003), upper arm muscle area (p = 0.045), fat-free mass (p = 0.025), and elbow breadth (p = 0.017) and greater skeletal maturation delay (p = 0.04). In older children (>9 years) low Znp was associated with decreased Tanner scores for pubic hair (p = 0.001) and breast and genital maturation (p = 0.009). No significant differences were seen in age, sex, or fat stores according to Zn status.
Conclusions:
Decreased plasma Zn is common in children with SS genotype sickle cell disease and is associated with decreased linear growth, skeletal growth, muscle mass, and sexual and skeletal maturation.